Published online before print August 1, 2005, doi: 10.1161/CIRCULATIONAHA.104.529800
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(Circulation. 2005;112:828-835.)
© 2005 American Heart Association, Inc.
Exercise Physiology |
Exercise Intolerance in Adult Congenital Heart Disease
Comparative Severity, Correlates, and Prognostic Implication
From the Adult Congenital Heart Program, Department of Cardiology, Royal Brompton Hospital (G.-P.D., K.D., W.L., S.V.B.-N., C.S.B., B.J., B.B., M.J.M., M.A.G.); Department of Clinical Cardiology, National Heart and Lung Institute, Imperial College (G.-P.D., D.O., P.A.P.-W.); and Cardiac Performance Unit, Department of Cardiology, St Mary’s Hospital (D.P.F.), London, UK.
Correspondence to Professor Michael A. Gatzoulis, MD, PhD, FACC, Adult Congenital Heart Program, Royal Brompton Hospital, Sydney St, SW3 6NP London, UK. E-mail m.gatzoulis{at}rbh.nthames.nhs.uk
Received December 15, 2004; revision received April 20, 2005; accepted April 28, 2005.
Background— Although some patients with adult congenital heart disease (ACHD) report limitations in exercise capacity, we hypothesized that depressed exercise capacity may be more widespread than superficially evident during clinical consultation and could be a means of assessing risk.
Methods and Results— Cardiopulmonary exercise testing was performed in 335 consecutive ACHD patients (age, 33±13 years), 40 non–congenital heart failure patients (age, 58±15 years), and 11 young (age, 29±5 years) and 12 older (age, 59±9 years) healthy subjects. Peak oxygen consumption (peak 
O2) was reduced in ACHD patients compared with healthy subjects of similar age (21.7±8.5 versus 45.1±8.6; P<0.001). No significant difference in peak O2 was found between ACHD and heart failure patients of corresponding NYHA class (P=NS for each NYHA class). Within ACHD subgroups, peak O2 gradually declined from aortic coarctation (28.7±10.4) to Eisenmenger (11.5±3.6) patients (P<0.001). Multivariable correlates of peak O2 were peak heart rate (r=0.33), forced expiratory volume (r=0.33), pulmonary hypertension (r=–0.26), gender (r=–0.23), and body mass index (r=–0.19). After a median follow-up of 10 months, 62 patients (18.5%) were hospitalized or had died. On multivariable Cox analysis, peak O2 predicted hospitalization or death (hazard ratio, 0.937; P=0.01) and was related to the frequency and duration of hospitalization (P=0.01 for each).
Conclusions— Exercise capacity is depressed in ACHD patients (even in allegedly asymptomatic patients) on a par with chronic heart failure subjects. Lack of heart rate response to exercise, pulmonary arterial hypertension, and impaired pulmonary function are important correlates of exercise capacity, as is underlying cardiac anatomy. Poor exercise capacity identifies ACHD patients at risk for hospitalization or death.
Key Words: exercise test • heart defects, congenital • heart failure • prognosis • survival
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